P-078: HEPATOBILIARY AND SPLENIC COMPLICATIONS OF SICKLE CELL DISEASE IN NIAMEY. A RETROSPECTIVE STUDY FROM 145 PATIENTS
Authors: MOUNKAILA B., GARBA M., BEIDARI A., TOURÉ I., AYOUBA TINNI H.
Journal: HemaSphere (2022)

Purpose: Sickle cell disease is the most widespread hereditary genetic disease in the world, particularly in black populations [1]. In Niger, there were 20% sickle cell trait and 1.8% were homozygous SS [2]. Subjects with major sickle cell disease may have serious clinical manifestations whose evolution will lead to numerous hepatobiliary and splenic complications variable in their severity and temporality. The objective of this work is to analyze the frequency and the characteristics of the hépatobiliary and splenic complications, in order to prevent them and to facilitate their taking up. Materials and methods: We led a retrospective and transverse study in the National Reference Center of sickle cell disease (CNRD) of Niamey between 2014 to 2021. The regularly followed patients were identified on the basis of medical file. On the whole 3000 files with a request of abdomino-pelvic ultrasound and hepatic enzymes had been consulted. Patients of less than 3, those of more than 50 years old and those whose medical file was incomplete are not included. Results: Approximately 4,8% patients (145 patients) presented complications to abdomino-pelvic ultrasound. The sex ratio (M/F) was of 1,15. The Average Age is 14,13 with extremes ranging from 4 to 49 years. The age bracket <10 years was more frequent (52,41%), followed by the one of 10 to 20 (27,01%). The sickle cell anemia phenotype consisted of 84,8% SS (123 cases), 14,5% of SC (21 cases) and 0,7% of Sβ° thalassaemia (1cases). The circumstances of the discovery of the disease are dominated by the splenomegaly and the vaso-occlusive crisis respectively in 22,8% and 15,2%. The hepatic enzyme aspartate amino transferase (ASAT) was high in 72,6% of the patients. The most frequent complications were hepatomegaly alone (HPM) (60,7%), followed by the hepato- splenomegaly in 17,3% and splenomegaly alone (SPM) in 9,66% of the cases. The vesicular lithiasis was found in 12,4% of the cases. The average age of patients with hepatomegaly and splenomegaly was 36,5 years old. The average age of the of onset of the lithiasis alone was 21,85 years old. Those with splenomegaly alone had an average of age younger 10,64. The average age of onset of the 3 associated complications hepatomegaly+ splenomegaly+ Lithiasis was 18,8 years old. Hyperbilirubinemia is higher among patients presenting hepatomegaly + Lithiasis, it is the same as for the hepatic enzymes. Indeed 73.79% of patients were hospitalized either because of vaso-occlusive crisis (35.42%) or anemia (21.53%). All the patients had received medical care with antibiotics, antimalarials, antispasmodics, analgesics and parenteral rehydration. Three patients had undergone cholecystectomy. Conclusion: Sickle cell disease is a serious public health problem in black african populations and major clinical findings despite anemia are liver and splenic complications which must been looked out and followed because of their morbidity and invalidity keeping home young pupils compromising their scholar education. A great carefull follow up should be done for these kind of patients. The authors do not declare any conflict of interest